[Anchoring proteins and cardiac sudden death: how and why?]

Med Sci (Paris). 2004 Apr;20(4):437-41. doi: 10.1051/medsci/2004204437.
[Article in French]

Abstract

Mutations in proteins responsible for ion transport in cardiac tissue can induce a destabilization of electrical function and provoke cardiac sudden death. Identification of a genetic anomaly in a French family that developed the syndrome of cardiac sudden death has revealed a crucial new element in normal cardiac electrical function : Ion channels need to be anchored to specific domains at the plasma membrane by an anchoring protein called ankyrin-B.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Action Potentials
  • Animals
  • Ankyrin Repeat
  • Ankyrins / chemistry
  • Ankyrins / deficiency*
  • Ankyrins / genetics
  • Ankyrins / physiology
  • Arrhythmias, Cardiac / etiology
  • Arrhythmias, Cardiac / genetics*
  • Arrhythmias, Cardiac / physiopathology
  • Calcium / physiology
  • Death, Sudden, Cardiac / etiology*
  • Electrocardiography
  • Heart Conduction System / physiopathology
  • Humans
  • Ion Channels / genetics
  • Ion Channels / physiology
  • Ion Transport / genetics
  • Long QT Syndrome / genetics
  • Long QT Syndrome / physiopathology
  • Mice
  • Mice, Knockout
  • Models, Biological
  • Sodium / physiology

Substances

  • ANK2 protein, human
  • Ank2 protein, mouse
  • Ankyrins
  • Ion Channels
  • Sodium
  • Calcium